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Pulmonary Atresia with Intact Ventricular Septum in a Neonate

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Pulmonary Atresia with Intact Ventricular Septum in a Neonate Indonesian Journal of Cardiology Indonesian J Cardiol 2017:38:218-25 pISSN: 0126-3773 / eISSN: 2620-4762 Case Report Pulmonary Atresia with Intact Ventricular Septum in a Neonate Emir Yonas1, Raymond Pranata2, Nuvi Nusarintowati3 , Introduction: Pulmonary atresia with an intact ventricular septum is a condition that is characterized by a complete obstruction to right ventricular outflow with varying degrees of right ventricular and tricuspid valve hypoplasia. This condition is uniformly fatal if untreated. In this case report, we present a case of a neonate with a pulmonary atresia with intact ventricular septum Case Presentation: A 2 days-old female Indonesian newborn was referred to our facility. The newborn was delivered from a G4P3A0 mother with a gestational age of 39 weeks (term delivery). Chest x-ray done at the referring facility is significant for a seemingly right heart hypertrophy, casting a “boot-shaped” appearance of the right heart border. Laboratory results done at the referring facility is significant for a neutrophilia of 82% and lymphocytopenia of 13% An episode of hypoglycemia was reported on referring facility with a blood sugar level of 50 mg/dl. Multiple episodes of cyanosis were reported at referring facility, CPAP was administered but the improvement was limited. Pulse oximetry averaged between 72-80% at the referring facility. Physical examina- tion shows an actively moving neonate, with a strong cry. Perioral cyanosis was seen. Vital signs were as follows; heart rate 128 x / minute, temperature 37oC, respiratory 1Faculty of Medicine, rate 54 x / minute, pulse oximetry 74%, capillary refill time < 3 seconds. The rest of YARSI University, the physical examination was within normal limits. Laboratory result on admission at Jakarta, Indonesia our facility is significant for low hematocrit, low erythrocyte count, leukopenia with 2Faculty of Medicine, neutrophilia and thrombocytopenia. Patient was admitted to NICU and given CPAP. Universitas Pelita Echocardiography 2 days upon arrival at our facility reveals a patent ductus arteriosus Harapan, Tangerang, with the pressure of 4-5 mmHg and diameter of 0.3 cm, no forward flow from the right Indonesia ventricle to the pulmonary artery, doppler mode demonstrated pulmonary artery filling 3Pediatric Cardiology from ductus arteriosus. The rest of the findings were within normal limits. division, University Conclusion: Pulmonary atresia with an intact ventricular septum is a condition that Of Indonesia, Jakarta, relies on the patent ductus arteriosus for pulmonary blood supply, the patency of it is Indonesia of paramount importance. (Indonesian J Cardiol. 2017;38:218-25) Keywords: Pulmonary Atresia, Patent Ductus Arteriosus, congenital heart disease, hypoxic spells, cyanosis 218 Indonesian J Cardiol • Vol. 38, Issue 4 • October - December 2017 Indonesian Journal of Cardiology Indonesian J Cardiol 2017:38:218-25 Laporan Kasus pISSN: 0126-3773 / eISSN: 2620-4762 Atresia Pulmonal dengan Septum Ventrikel Intak pada Neonatus Emir Yonas1, Raymond Pranata2, Nuvi Nusarintowati3 , PENDAHULUAN: Atresia arteri pulmonalis dengan septum ventrikel yang utuh merupakan kondisi yang dikarakterisasikan dengan sumbatan total pada right ventricular outflow dengan berat hipoplasia ventrikel kanan dan trikuspid kanan yang beratnya bervariasi. Pada umumnya kondisi tersebut fatal apabila tidak ditangani. Pada laporan kasus ini, kami melaporkan suatu kasus neonates dengan atresia arteri pulmonalis dengan septum ventrikel yang utuh. PRESENTASI KASUS: Neonatus perempuan berumur 2 hari dirujuk ke rumah sakit kami. Bayi dilahirkan dari G4P3A0 usia kehamilan 39 minggu. Ronsen thoraks yang dilakukan oleh fasilitas kesehatan yang merujuk memperlihatkan hipertrofi jantung kanan serta bentuk “boot- shaped” pada batas jantung kanan. Hasil laboratorium pada fasilitas kesehatan tersebut menunjukan neutrofilia dan limfositopenia. Terdapat juga riwayat hipoglikemi. Episode sianosis berulang terjadi pada fasilitas kesehatan yang merujuk. Pemasangan CPAP kurang membuahkan hasil dengan saturasi O2 72-80% ketika tiba pada rumah sakit kami. Pemeriksaan fisik menunjukan tangis kuat dan gerak aktif dengan sianosis perioral. Detak jantung 128 kali/menit, suhu 37oC, laju naas 54 kali/menit, oksimetri nadi 74% dan waktu isi kapiler <3 detik. Pemeriksaan laboratorium saat pasien dating menunjukan hematocrit, hitung eritrosit, leukosit dan thrombosit yang rendah. Pasien dirawat di NICU dan diberikan CPAP. Ekokardiografi 2 hari kemudian menunjukan duktus arteriosus patent dengan tekanan 4-5 mmHg dan diameter 0.3 cm, tidak ada aliran maju dari ventrikel kanan kepada arteri pulmonalis, mode Doppler menunjukan pengisian arteri pulmonalis dari duktus arteriosus. Pemeriksaan lainnya dalam batas normal. KESIMPULAN: Atresia arteri pulmonalis dengan septum ventrikel yang utuh merupakan suatu kondisi dimana aliran darah menuju paru bergantung pada duktus arteriosus yang paten sehingga patensi daripada duktus tersebut sangatlah penting. (Indonesian J Cardiol. 2017;38:218-25) Kata Kunci: Atresia pulmonalis, duktus arteriosus paten, penyakit jantung bawaan, periode hipoksik, sianosis Introduction Pulmonary atresia with an intact ventricular septum is a condition that is characterized by a complete obstruc- 1Fakultas Kedokteran, Universitas YARSI, Jakarta, Indonesia, tion to right ventricular outflow with varying degrees 2Fakultas Kedokteran, Universitas Pelita Harapan, Tangerang, of right ventricular and tricuspid valve hypoplasia. As 3 Indonesia, Divisi Kardiologi Pediatrik, Universitas Indonesia, a result of these defects, blood is thus unable to flow Jakarta, Indonesia from the right ventricle to the pulmonary artery and Correspondence: lungs, and an alternative source of blood flow is then Dr.Emir Yonas, Facult of Medicine, YARSI University, Jakarta, required for survival. This condition is uniformly fatal Indonesia. E-mail: [email protected] if untreated. Indonesian J Cardiol • Vol. 38, Issue 4 • October - December 2017 219 Indonesian Journal Of Cardiology In this case report, we present a case of a neonate with cytopenia of 13% (normal range for individuals < 1 a pulmonary atresia with intact ventricular septum years old: 20-70%), the rest of laboratory values are within normal limits. An episode of hypoglycemia was reported on Case Presentation referring facility with a blood sugar level of 50 mg/ dl, subsequently, the neonate was given dextrose 10% A female Indonesian newborn was referred to our facility. with normal saline. No further episodes are reported. Due to suspicion of cyanotic heart disease. The newborn Multiple episodes of cyanosis were reported at referring was delivered 2 days prior to referral to our facility via facility, CPAP was administered but the improvement cesarean section. The newborn was delivered from a was limited. Pulse oximetry averaged between 72-80% G4P3A0 mother with a gestational age of 39 weeks (term at the referring facility. delivery). Weight on delivery was 2500 grams, body Upon arrival at our institution, a physical examina- length 47 centimeters, with an Apgar score at 1 and 5 tion shows an actively moving neonate, with a strong minutes of 7 and 9 respectively. She was referred to our cry. Perioral cyanosis was seen. Vital signs were as fol- facility aged 48 hours. An inquiry of the gestational his- lows; heart rate 128 x / minute, temperature 37oC, tory reveals a term, appropriate for gestational age fetus. respiratory rate 54 x / minute, pulse oximetry 74%, Maternal age at current pregnancy was 38 years old. The capillary refill time < 3 seconds. Cardiac auscultation mother had 1 vaginal delivery and 2 cesarean section pre- reveals single and decreased second heart sound, with viously. The mother denied any history of sickness during no other murmur audible. The rest of the physical pregnancy. Patient’s parents denied any history of illnesses examination was within normal limits. of the heart and lungs in the family. Chest x-ray done at Laboratory result on admission at our facility is the referring facility is significant for a seemingly right significant for low hematocrit of 37% (normal range heart hypertrophy, casting a “boot-shaped” appearance 42-60%), low erythrocyte count of 3.8 mil/ microliter of the right heart border. Assessment of lung vasculature (normal range 3.9-5.5 mil/microliter), leukopenia with on CXR reveals an oligemic lung. WBC count of 3190 (normal range 9000-30,000 / The neonate was given intramuscular vitamin K, microliter), thrombocytopenia with count of 142000 ophthalmic antibiotic drops, normal saline intrave- / microliter (normal range 150,000-400,000 / micro- nously and cefotaxime (2x 125 mg, 4 doses in total) liter). Differential counting of white blood cell showed at referring facility. neutrophilia of 73% (normal range 50-70%). Laboratory results done at the referring facility is A suspicion of PAIVS was established by physical significant for a neutrophilia of 82 % (normal range examination and chest X ray, the neonate was then for individuals < 1 years old: 17-60%) and lympho- admitted to our neonatal intensive care unit. Continu- Figure 1. Patient’s echocardiography showing patent ductus Figure 2. Patient’s echocardiography showing patent ductus arteriosus filling from the aorta arteriosus width 220 Indonesian J Cardiol • Vol. 38, Issue 4 • October - December 2017 Yonas E et al: Pulmonary Atresia with Intact Ventricular Septum in a Neonate ous positive airway pressure was administered with a PAIVS is an acquired lesion due to perturbation
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